12/7 - A Scary Day

Around 9AM this morning Emily's oxygen saturation started dropping... fast. We have dealt with these episodes before, so we felt prepared for another little event. They are always intense, but not like this morning. We skipped the Chest Physiotherapy (CPT) and went straight for the In-Exsufflator Cough Machine (aka Cofflator or Cough Assist) and cranked up the BiPAP machine for support between coughs, all the while suctioning any secretions that would come up. These episodes that come on so suddenly are caused by mucus plugs that shimmy their way into an area of the lung causing, in some cases, extremely fast desaturations. SMA kids often die this way. Emily's sats kept dropping while I was using the Cough Assist and suctioning in between sets. The last number we saw on the Pulse Oximeter was 11%. To give you a frame of reference -- if her sats drop below 92% we worry.

When I get nervous my voice shakes. When I am terrified my hands shake. My hands were shaking badly this morning.

At this point Emily was completely unresponsive. I had watched her eyes roll back into her head as I was calling her name, then she stopped breathing. We pulled out our Ambu bag (resuscitator ) and started giving her breathes with 6 Liters of oxygen attached to the other end. For about 5-10 mins we had her "bagged" like this, making her breath. During this time her oxygen saturation started to climb ever so slowly. After jumping up into the 80% range I started with the cough assist and suctioning again. This went on for maybe 30 mins. We were listening to her lungs with the stethoscope and there was very little, if any, air movement through her right lung. We feared another collapse.

During that time we switched from Cough Assist to BiPAP and gave her CPT. With Albuterol nebulizer treatments, CPT, Cough Assist, Positioning, and BiPAP we were able to recover her to a somewhat stable condition, but she was still requiring 6 Liters of Oxygen to keep her around 90%. At this point we could also hear more air movement in her right lung, but we knew we had to make a decision:
  1. Take her to the ER to be re-intubated
  2. "Let her go"
  3. Keep working on her the way we were and hope to get her stable
We had a few conversations with other SMA parents, but chose to keep working on her via a protocol that we are familiar with:
  1. CPT 10-15 mins
  2. Cough Assist 4 sets of 5 breaths
  3. Postural drainage
  4. Cough Assist 4 sets of 5 breaths
  5. Continue until able to sat above 92%
After about another hour of this Emily started sating well in the 90's and we turned her oxygen down little by little until we were at 0 Liters. She finally stabilized around 98% with NO oxygen and her lung was sounding much better. Amazing!

Once she was stable we started trying to figure out what we wanted to do. We needed to know more about what happened and the only place to do that was at the ER where they could take an X-ray to see if that lung was collapsed or not. We were reluctant to go b/c we didn't want her back in the PICU, but we really needed to see a Pulmonologist. We consulted the Pediatric Pulmonology team at Johns Hopkins and Emily's Pediatrician (also a Johns Hopkins Dr.) and decided it would be best to take her in, with full expectations of being seen by the Pulmonologists and her Dr. before any other decisions were made about her care.

Emily was stable and happy - two things we did not go to the ER with nearly four weeks ago. This visit was good. We saw the Pulmonologists and told them the story. I could see the grin forming on their faces when they said, "you guys did everything perfectly! The only complaint we have is that it wasn't taped because it would have been a perfect teaching tool for every Dr. in this hospital!" They were very pleased to hear how everything was handled and, let me tell you, we needed that encouragement after the last few hours, especially after the last few weeks in the PICU.

We have since made it back home and Emily is as happy as ever. The Docs helped us tweak Emily's machines a little to suit her needs better and we all agreed that back home is where we needed to be. We left with an inhalant steroid for Emily's lungs and she was very dehydrated (not a good thing AT ALL for SMA kids) so we are giving her more fluids. Oh yeah, her chest X-ray looked better than the day we were discharged from the PICU so things are looking very good! She still had junk in her right lung, but we are working on that... around the clock. We are still not able to have nursing covered by my insurance, so alone we will be until we can figure out a way to get shift nursing.

So, that's the story. Things learned:
  1. Most every SMA family needs a GOOD Pulmonology team on their side and the Johns Hopkins Pediatric Pulmonary team is a good one. They even support more aggressive care with Type I kids and that can be very difficult to come by.
  2. We are grateful for our incredible Pediatrician, Dr. Dudas - SMA families need one of these too
  3. With these two things SMA families can feel comfortable taking their child to the ER
  4. Diana's two cents - MOST IMPORTANTLY -- We learned that Diana and I are a GREAT team for Emily. We fall into our roles and get things done pretty flawlessly. We are grateful for each other's unique abilities and we cling to them. (Thanks for trusting me Diana!)
Thanks for reading.


That is awesome!! Scary and I know you must be worn out both physically and emotionally - but how wonderful to know - I mean really really know - that you know how to take care of your own child and that you can give her the best care! Every parent doubts that at times...
We will continue to pray that the Spirit would give you the wisdom and endurance that you need. He is SO good to His children!!
Stephen Goddard said…
I just discovered your blog yesterday after someone left a message for me on my blog, http://kaylieelizabeth.blog.com

Six weeks ago my daughter was sent to the ER and then to the PICU for respiratory distress. We are now on day 47 in the hospital and Kaylie has been diagnosed with SMA type 2. I've been keeping a daily blog since day four.

We've been having a hard time getting her weaned from 24/7 bipap. One of the fears I would say has been what would I do if she crashed at home like she has at the hospital. I'm encouraged reading your story and hope my wife and I could respond as well as you did.

Stephen G.